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Indian Pediatr 2012;49: 854-855

Rowell Syndrome


LS Solanki, M Dhingra and GP Thami

Department of Dermatology D-Block, 5th Floor, Govt medical college and Hospital,
Sector-32, Chandigarh-160030, India.
Email: [email protected]
 



A 13-year-old female child presented with multiple erythematous papules and plaques with some of them showing targetoid appearance (Fig.1a), along with a single large 8 cm x 10 cm sized polycyclic, erythematous plaque, involving right side of face, upper neck and external ear area (Fig.1b). Oral mucosa showed erythematous plaque involving right side hard palate with overlying multiple superficial erosions (Fig.1c). She had history of low-grade fever, photosensitivity, malar rash, Raynaud’s phenomenon, chilblains and recurrent oral ulceration since 6 months. There was no history suggestive of recent infection or drug intake prior to onset of lesions.

Fig.1 Rowell syndrome

Laboratory studies showed positive antinuclear antibodies (ANA) in a speckled pattern at 1:160 dilutions, ESR 25 mm/I/h, and positive rheumatoid factor. dsDNA antibody, AntiRo and antiLa antibodies were negative. Hemogram, C3, C4, urine routine, urinary 24-hr protein, and renal function test were all within normal limits. Electrocardiogram and chest X-ray were normal. Histopathology of skin lesion was consistent with the diagnosis of systemic lupus erythematosus (SLE). Patient was advised photo protection and started on tapering oral corticosteroid along with Hydroxy-chloroquine. Lesions resolved after one month of treatment leaving only mild pigmentary changes with no relapse at 6-month follow up.

Rowell Syndrome (RS) is a unique clinical association of lupus erythematosus (LE) with erythema multiforme (EM) like lesions and a characteristic immunologic pattern. The etiopathogenesis is unknown. Diagnostic criteria include three major criteria: (1) LE (systemic, discoid, or subacute); (2) EM-like lesions with or without mucosal involvement; and (3) speckled ANA; and three minor criteria: chilblains, antiRo or antiLa antibodies, and positive rheumatoid factor. For diagnosis all three major and at least one minor criterion should be fulfilled. Children with LE may present with EM-like lesions which must be differentiated from Classical EM which is often precipitated by factors such as infective agents or drugs and is not associated with any specific serological abnormalities or with chilblains. Treatment is with topical and oral steroids, dapsone, and antimalarial agents but response is variable with frequent recurrences.

 

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